POS1346 PREVALENCE, PHENOTYPICAL CLINICAL CLUSTERS AND TREATMENT OF NEUROBEHÇET’S DISEASE. STUDY IN NORTHERN SPAIN
Authors: A. Herrero-Morant, C. Álvarez-Reguera, L. Sanchez-Bilbao, D. Martínez-López, J. L. Martín-Varillas, G. Suárez-Amorín, R. Fernández Ramón, M. C. Mata Arnaiz, M. Á. González-Gay, R. Blanco
Journal: Annals of the Rheumatic Diseases (2022)

Background Behçet’s disease (BD) may present with different clinical phenotypes. Ocular and Neurobehçet’s Disease (NBD) are severe complications [1-4]. Data on NBD epidemiology, clinical phenotype and therapy are scarce and controversial. Objectives In a wide unselected single-center series of BD our aims were to assess a ) NBD prevalence, b ) associations with other clinical clusters and c ) treatment. Methods Cross-sectional study of all 120 patients diagnosed with BD in Northern Spain, between January 1, 1999 to December 31, 2019. Finally, 96 patients were included in this study according to 2014 International Criteria for Behçet Disease (ICBD) [5]. NBD was diagnosed according to the International Consensus Recommendation (ICR) criteria [4]. Results NBD was diagnosed in 23 of 96 (24%) patients (15 women/8 men) (mean age: 44±13.9 years). NBD was classified as parenchymatous (n=10, 43.5%), non-parenchymatous (n=10, 43.5%) and mixed (n=3, 13%). HLAB51 was positive in 5 out of 13 (38.4%) patients tested. The main cluster of clinical associations were oral aphthae (n=20, 87%); ocular (n=14, 60.9%), cutaneous (n=10, 43.5%), articular (n=9, 39.1%), vascular (n=4, 17.4%) and intestinal (n=1, 8.7%) involvement (Figure 1). Figure 1. Clusters of clinical associations of NBD Treatments were oral corticosteroids (n=16; 69.6%; mean maximum dose 42±12.5 mg/ day, conventional immunosuppressants (n=13, 56.5%) and Biological Therapy (BT) (n= 7; 30.4%). BT was used in patients who were refractory to conventional immunosuppressants. Monoclonal anti-TNFα were used as the first option in all patients who received BT. In 3 out of 7 (42.7%) patients BT was switched due to inefficacy. Table 1 shows the main NBD clinical subtypes and treatment. Table 1. Main clinical features and treatment of 23 patients with NBD n (% ) Mean maximum oral prednisone dose, (SD) mg/day Conventional immunosuppressants, n (% ) monoclonal anti-TNFα, n (% ) Tocilizumab, n (% ) Anakinra, n (% ) Parenchymal phenotype 10 (43.5 ) 51.7±19.3 6 (46.2 ) 4 (57.1 ) 0 0 - Hemiparesis 5 (50 ) 52.5±7.5 2 (50 ) 3 (75 ) - Optic neuropathy - Encephalopathy 3 (30 ) 1 (10 ) 52.3±26.345 2 (66.7 ) 1 (16.7 ) 00 - Ophtalmoparesis 1 (10 ) 0 1 (16.7 ) 1(25 ) Non-parenchymal phenotype 10 (43.5 ) 42±12.5 5 (38.5 ) 2 (28.6 ) 0 0 - Aseptic meningitis 10 (43.5 ) 42±12.5 5 (38.5 ) 2 (28.6 ) Mixed 3 (13 ) 45±15 2 (15.4 ) 1(14.3 ) 1 (14.3 ) 1 (14.3 ) - Aseptic meningitis and ophtalmoparesis 1 (33.4 ) 60 0 0 0 0 - Aseptic meningitis and other cranial nerve involvement 1 (33.4 ) 0 1 (50 ) 0 0 0 - Encephalopathy and intracranial hypertension 1 (33.4 ) 30 1(50 ) 1(100 ) 1(100 ) 1(100 ) Complete remission was achieved in 18 of 23 cases (78.2%), partial response in 2 out of 23 cases (8.7%). No severe adverse effects were observed. Conclusion NBD was observed in 24% of patients with BD. The most frequent clinical clusters of NBD were oral aphthae and ocular involvement. All patients treated with either conventional immunosuppressant or BT achieved clinical remission. References [1]Martín-Varillas JL, et al. Ophthalmology 2018 Sep;125(9):1444-1451. doi: 10.1016/j.ophtha.2018.02.020. [2]Atienza-Mateo B, et al. Arthritis Rheumatol 2019 Dec;71(12):2081-2089. doi: 10.1002/art.41026. [3]Santos-Gómez M, et al. Clin Exp Rheumatol 2016 Sep-Oct;34(6 Suppl 102): S34-S40. [4]Kalra S, et al. Diagnosis and management of Neuro-Behçet’s disease: international consensus recommendations. J Neurol. 2014 Sep;261(9):1662–76. [5]International Team for the Revision of the International Criteria for Behçet’s Disease (ITR-ICBD). J Eur Acad Dermatol Venereol. 2014 Mar;28(3):338-47. Disclosure of Interests Alba Herrero-Morant: None declared, Carmen Álvarez-Reguera: None declared, Lara Sanchez-Bilbao: None declared, David Martínez-López: None declared, José Luis Martín-Varillas Grant/research support from: AbbVie, Pfizer, Lilly, Janssen, UCB, and Celgene, Guillermo Suárez-Amorín: None declared, Raúl Fernández Ramón: None declared, M. Cristina Mata Arnaiz: None declared, Miguel Á. González-Gay Speakers bureau: Abbvie, Roche, Sanofi, Lilly, Celgene, Sobi, and MSD, Grant/research support from: Abbvie, MSD, Janssen, and Roche, Ricardo Blanco Speakers bureau: Abbvie, Lilly, Pfizer, Roche, BMS, Janssen, and MSD, Grant/research support from: Abbvie, MSD, and Roche